Pulmonary arterial hypertension (PAH), a subset of pulmonary hypertension (PH), is characterized by resistance to blood flow through the lungs. Higher right ventricle pressure may eventually progress to heart failure.

The symptoms of PAH are nonspecific and develop slowly (and are often confused with signs of aging or being in poor shape). The mean age of diagnosis ranges from 36 to 65 years, but also note that penetrance is low (though higher in females than males).

Current therapy improves clinical function, but has a modest effect on survival.

Individuals at risk for PAH should undergo aggressive treatment for any existing conditions that contribute to the development or worsening of PH (e.g., sleep apnea and systemic hypertension).

Recommendations for screening vary from annual echocardiography to ongoing clinical monitoring for the development of symptoms that would signal disease progression and warrant initiation of pharmacotherapy.